Rheumatic Fever and Rheumatic Heart Disease

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					 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi
                Rheumatic Fever and Rheumatic Heart Disease

                               Dr A.A. Adebiyi

                                Cardiology Unit
                               College of Medicine
                               University of Ibadan
                                     Ibadan


                               October 7, 2010
                Rheumatic Fever

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi
                Aetiology

 Rheumatic
 Fever and
 Rheumatic          autoallergic disease
Heart Disease

  Dr A.A.           triggered by infection with some strains of streptococcal
  Adebiyi
                    pyogenes
                    express antigens which cross-reacts with those of human
                    connective tissues
                    Affects children and young adults
                    definite family variation in susceptibility
                    Prevalence in developed countries had progressively
                    declined—most likely due to improvement in housing and
                    hygienic conditions
                    The clinical manifestations of acute RF follow a group A
                    streptococcal (GAS) infection of the tonsillopharynx after
                    a latent period of approximately 3 weeks.
                Rheumatic fever (RF)

 Rheumatic
 Fever and          The major importance of acute RF is its ability to cause
 Rheumatic
Heart Disease       fibrosis of heart valves, leading to crippling haemodynamic
  Dr A.A.
  Adebiyi
                    of chronic heart disease.
                    most common cause of acquired heart disease in children
                    and young adults worldwide.
                    incidence of RF has declined in many developed countries
                    but still remains a major problem in developing countries.
                    Initial attacks of RF occur most commonly between the
                    ages of 6 and 15 years
                    RF rarely occurs before the age of 5 years
                    risk of RF is increased in populations at high risk for
                    streptococcal pharyngitis,
                        such as military recruits
                        persons living in crowded conditions
                        those in close contact with school-age children.
                Diagnosis

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi

                    mainly clinical
                    requires supporting evidence from clinical microbiology
                    and clinical immunology laboratories.
                    Jones(1944) proposed criteria to assist in standardizing
                    the diagnosis of RF
                    modified in 1992 by the American Heart Association
                The Jones Criteria for Rheumatic Fever,
                Updated 1992
 Rheumatic
 Fever and
 Rheumatic
Heart Disease
                    Major Criteria
  Dr A.A.
  Adebiyi               Carditis
                        Migratory polyarthritis
                        Sydenham’s chorea
                        Subcutaneous nodules
                        Erythema marginatum
                    Minor Criteria
                        Clinical
                             fever
                             Arthralgia
                        Laboratory
                             Elevated acute phase reactants
                             Prolonged PR interval
                carditis of ARF

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.           pancarditis involving the pericardium, myocardium, and
  Adebiyi
                    endocardium.
                    40 and 60% of patients with ARF have evidence of carditis
                    characterised by
                        sinus tachycardia,
                        murmur of mitral regurgitation
                        S3 gallop
                        pericardial friction rub
                        cardiomegaly
                        prolonged PR interval and evidence of heart failure may be
                        present – nonspecific
                carditis of ARF

 Rheumatic
 Fever and          Healing of the rheumatic valvulitis
 Rheumatic
Heart Disease           leads cause fibrous thickening and adhesion,
  Dr A.A.               resulting in the most serious complication of rheumatic
  Adebiyi
                        fever, i.e., valvular stenosis and/or regurgitation
                    mitral valve is involved most frequently, followed by the
                    aortic valve.
                    isolated aortic valve disease as a consequence of acute
                    rheumatic fever is quite rare
                    patients with aortic valve disease due to rheumatic fever
                    also have involvement of the mitral
                    minor degrees of rheumatic valvular involvement can lead
                    to susceptibilities to infective endocarditis
                    rheumatic pericarditis can cause a serous effusion, fibrin
                    deposits, and even pericardial calcification,
                        it does not lead to constrictive pericarditis.
                migratory polyarthritis

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi           present in as many as 75% of cases,
                    affecting the ankles, wrists, knees, and elbows over a
                    period of days
                    does not affect the small joints of the hands or feet and
                    seldom involves the hip joints
                    The difference between arthralgia (subjective joint pain)
                    and arthritis (joint pain and swelling) must be understood.
                    occassionally arthralgia is used (incorrectly) as a major
                    criterion
                Sydenham’s chorea

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi



                    occurs in fewer than 10% of patients with rheumatic fever.
                    The latent period between the onset of the initiating
                    streptococcal infection and the onset of Sydenham’s
                    chorea may be as long as several months
                Subcutaneous nodules and erythema marginatum

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi           are rare major manifestations
                    usually present in fewer than 10% of cases.
                    Subcutaneous nodules are found over extensor surfaces of
                    joints, are seen most often in patients with long-standing
                    rheumatic heart disease,
                    extremely rare in patients experiencing an initial attack.
                    Erythema marginatum is an uncommon manifestation. It
                    is an evanescent macular eruption with rounded borders
                    usually concentrated on the trunk
                To fulfill the Jones criteria

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.           either two major criteria,
  Adebiyi
                         or
                         one major criterion and two minor criteria,
                         plus
                         evidence of an antecedent streptococcal infection are
                         required.
                              recovery of the organism on culture
                              or
                              by evidence of an immune response to one of the
                              commonly measured group A streptococcal antibodies
                              (e.g., anti-streptolysin O, anti-deoxyribonuclease B,
                              anti-hyaluronidase).
                diagnosis

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi

                    group A streptococci can be recovered from the URT of
                    only 25 to 40% of patients at the time of diagnosis
                        two or three throat cultures should be obtained before
                        antibiotic therapy
                    80% of patients with ARF have an elevated ASO titer at
                    presentation
                diagnosis

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi
                    sensitivity increases to 95% with anti-deoxyribonuclease B,
                    anti-hyaluronidase
                    demonstration of a rising titer from the acute to the
                    convalescent phase
                        more reliable means of documenting the recent infection
                    the diagnosis should be reconsidered if three antibody
                    tests are negative and there is no evidence of a preceding
                    infection
                treatment

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.           mainly
  Adebiyi
                        anti-streptococcal antibiotic therapy
                        therapy for the clinical manifestations of the disease
                    at diagnosis
                        patients should be treated as if they have GAS–
                        irrespective of culture status
                             complete 10-day course in adults of either oral penicillin V
                             (500 mg twice daily),
                             erythromycin (250 mg four times daily) for those with
                             penicillin allergy.
                             intramuscular benzathine penicillin G - a single
                             intramuscular injection of 1.2 million units
                secondary prophylaxis

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi           initiated to prevent subsequent colonisation of the upper
                    respiratory tract with group A streptococci.
                    Recommendations of the American Heart Association and
                    of the World Health Organisation
                        are for intramuscular injection of 1.2 million units of
                        benzathine penicillin G every 4 weeks
                        or for oral penicillin V (250 mg twice daily) or oral
                        sulfadiazine (1.0 g daily)
                    secondary prophylaxis should be given for a five years after
                    the diagnosis or till age of 21 years
                Medical therapy for the manifestations of ARF

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi
                    depends on the clinical status of the patient
                    with arthritis
                        salicylates in doses escalating to 2 g four times daily
                        are very effective
                        Salicylates may be given for 4 to 6 weeks and gradually
                        tapered so as to prevent a rebound.
                        The erythrocyte sedimentation rate is used to determine
                        the rate of taper for salicylates.
                Medical therapy for the manifestations of ARF

 Rheumatic
 Fever and          with significant carditis
 Rheumatic
Heart Disease
                        steroid therapy are particularly effective and may be life
                        saving in very ill patients
  Dr A.A.
  Adebiyi                    prednisolone 1 to mg/kg/d
                        no evidence that steroid therapy affects the course of
                        carditis or diminishes the incidence of residual heart disease
                        conventional medical measures for heart failure if CHF
                    previously patients with acute rheumatic fever were kept
                    at complete bed rest for months.
                        now, indication for complete bed rest include
                             persistent active carditis
                             severe heart failure.
                        Patients with arthritis will begin to feel better very soon
                        after anti-inflammatory therapy with salicylates is begun.
                             released from bed rest then, but
                        should not resume full activity
                             until signs of inflammatory process have abated
                             acute-phase reactants – returned to normal
                MITRAL STENOSIS

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi
                   Obstruction of flow from left atrium to left ventricle
                   because of a narrowed mitral orifice.
                   almost always caused by previous rheumatic
                   feverRheumatic fever cause
                       a chronic process of valvular fibrosis
                       fusion
                       calcification with shortened, thickened chordae tendineae.
                   A left atrial myxoma can also cause obstruction at the
                   mitral orifice.
                   rarely congenital
                       Infants with isolated congenital MS rarely live beyond 2 yr
                       unless the MS is supravalvular.
                Symptoms

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.          In temperate climates symptoms of MS usually begin
  Adebiyi
                   between ages 30 and 40.
                   In tropical or subtropical countries, progression is swifter,
                   often occurring in childhood.
                   The first symptoms of MS are usually exertional dyspnoea
                   or fatigue.
                   Frank pulmonary oedema occurs with sudden elevations of
                   left atrial pressure (e.g., when uncontrolled atrial
                   fibrillation produces too rapid a ventricular rate as an
                   added insult to the loss of atrial contraction).
 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.       Haemoptysis due to rupture of small pulmonary vessels
  Adebiyi
                pulmonary oedema is likely to occur with the increased
                blood volume in pregnancy.
                Emboli can occur in up to 15% of patients and are usually
                associated with atrial fibrillation but can occur in sinus
                rhythm.
                Left vocal cord paralysis (Ortner’s syndrome) can cause
                huskiness due to paralysis of the left recurrent laryngeal
                nerve, which is compressed when a dilated left atrium
                presses it against a dilated pulmonary artery.
                Signs

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi
                   malar flush (mitral facies) is seen only with low CO and
                   severe pulmonary hypertension.
                   Left parasternal heave due to RV hypertrophy and dilation
                   or to a normal RV held against the sternum by a large left
                   atrium.
                   The S1 at the apex and the S2 may be palpable
                   Auscultation reveals a slightly exaggerated P2 with a
                   normal split.
                Signs

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
                   An opening snap, loudest between the apex and left lower
  Adebiyi          sternal border, can be heard if the mitral valve is not
                   excessively fibrosed and calcified
                   apical diastolic rumbling murmur, usually with a
                   presystolic crescendo (if still in sinus rhythm) over a
                   palpable apex beat (
                   Silent MS may occur in the presence of atrial fibrillation.
                   A soft decrescendo diastolic murmur along the left sternal
                   border may be due to pulmonary regurgitation (Graham
                   Steell’s murmur) secondary to severe pulmonary
                   hypertension
                Diagnosis

 Rheumatic
 Fever and
 Rheumatic
                   ECG
Heart Disease
                         P mitrale
  Dr A.A.
  Adebiyi
                         right axis deviation due to RV hypertrophy
                         RVH
                   X-ray
                         straightening of the left cardiac border due to a dilated left
                         atrial appendage.
                         The main pulmonary artery, or trunk is prominent,
                         The upper lobe pulmonary veins may be dilated–a
                         redistribution of blood flow from the lower to upper lobes
                         due to compression of the lower lobe veins.
                         double shadow of a fibrosed, enlarged left atrium is
                         characteristic along the right cardiac border.
                         Kerley B lines are horizontal lines in the lower posterior
                         lung fields diagnostic of interstitial oedema associated with
                         a high left atrial pressure.
                Diagnosis

 Rheumatic
 Fever and
 Rheumatic
Heart Disease
                   Echocardiography
  Dr A.A.
  Adebiyi              shows valvular calcification
                       the patient’s suitability for valvotomy
                       the size of the left atrium.
                       detect associated mitral regurgitation.
                       exact area of the mitral orifice.
                   Cardiac catheterization
                       determines the wedge pressure (reflecting left atrial
                       pressure)
                       indicates the degree
                            of pulmonary hypertension,
                            of MS,
                            and of regurgitation
                Prophylaxis and Treatment

 Rheumatic
 Fever and         asymptomatic patients,
 Rheumatic             penicillin is used prophylactically for streptococcal
Heart Disease

  Dr A.A.
                       infections
  Adebiyi              IE prophylaxis for surgical procedures.
                   symptomatic patients
                       β-blockers or Ca blockers to slow heart rate
                       digitalis and diuretics are given
                       Anticoagulants are recommended
                       Antiplatelet drugs (e.g., aspirin) may be substituted when
                       warfarin is contraindicated
                   Surgery for patients who remain symptomatic despite
                   medical management.
                   Critical MS requiring valvotomy or valve replacement is
                                                          ´
                   associated with an oval orifice ¡= 1.75 d7 0.85 cm.
                       balloon valvuloplasty
                       open valvotomy and valvuloplasty can be done.
                       valve replacement is necessary
                MITRAL REGURGITATION

 Rheumatic
 Fever and
 Rheumatic        Retrograde flow from the left ventricle through an
Heart Disease
                  incompetent mitral valve into the left atrium.
  Dr A.A.
  Adebiyi         The most common causes in adults
                      rheumatic valve damage
                      myxomatous degeneration with or without MVP
                      papillary muscle dysfunction
                      ruptured chordae tendineae.
                      Rare causes
                           left atrial myxoma
                           endocardial cushion defect with a cleft anterior leaflet
                           SLE
                           markedly calcified mitral annulus (mainly in elderly women
                      Pure rheumatic MR
                           due to shortening of valve cusps and of papillary muscles
                           chordae tendineae that become matted and adherent to
                           the valve.
                MITRAL REGURGITATION

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
  Adebiyi         Symptoms
                     Severe MR may cause palpitations long before heart failure
                     symptoms develop
                     If MR is severe, the high left atrial pressure can produce
                     dyspnoea due to the high regurgitant wave (CV wave)
                     even before heart failure symptoms of low cardiac output
                     (CO) occur; rarely
                     hoarseness due to Ortner’s syndrome may occur
                     Atrial fibrillation is common when the left atrium enlarges
                     and is certain when it is extremely large.
                Physical examination of moderate to severe
                MR
 Rheumatic
 Fever and
 Rheumatic
                    brisk pulse
Heart Disease
                    sustained left parasternal movement due to expansion of
  Dr A.A.
  Adebiyi           an enlarged left atrium
                    sustained and enlarged apical area movement displaced to
                    the left.
                    On auscultation, the second heart sound (S2) is usually
                    widely
                    A pansystolic murmur is loudest at the apex
                    Severe silent MR with an absent or soft murmur
                         acute MI
                         endocarditis
                         severe dilated cardiomyopathy with congestive heart failure
                         combined MR and stenosis
                         S3 at the apex will be loud in proportion to the degree of
                         MR
                Diagnosis

 Rheumatic
 Fever and
 Rheumatic
                   CXR
Heart Disease            dilatedleft atrium and ventricle
  Dr A.A.
  Adebiyi
                         Pulmonary vascular congestion
                   ECG
                         left atrial and ventricular hypertrophy with or without
                         ischemia.
                   Echo
                         Marked separation of the septal echo from the mitral valve
                         echo strongly suggests LV dilatation and depressed LV
                         contractility.
                         Doppler echocardiography, especially color Doppler, can
                         quantify the
                         Many causes of MR give diagnostic echocardiographic
                         evidence (eg, a dilated ventricle, prolapse, myxomas,
                         ruptured chordae, segmental wall motion abnormalities,
                         calcified annulus
                Prophylaxis and Treatment

 Rheumatic
 Fever and
 Rheumatic
Heart Disease

  Dr A.A.
                   Prophylaxis against endocarditis
  Adebiyi
                   If the cause is rheumatic and the MR is at least
                   moderately severe, rheumatic fever prophylaxis
                   To prevent pulmonary and systemic emboli
                       anticoagulation should be used in patients with gross heart
                       failure or with atrial fibrillation.
                   early valve replacement increases the chance of a good
                   outcome and decreases the chance of worsening LV
                   function.
                   Valve reconstruction is an alternative with low
                   perioperative mortality and good long-term prognosis.

				
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